Life-threatening bleeding in a patient with pemphigoid-induced acquired hemophilia A and successfully treated with rituximab and rFVIIa A case report

Rationale: Acquired hemophilia A (AHA) is a rare bleeding disorder with prolonged activated partial thromboplastin time (aPTT). Severe hemorrhage may occur, especially in refractory AHA. Patient concerns: We reported a 63-year-old man who suffered from life-threatening bleeding after the onset in lower limbs. Diagnoses: The patient was diagnosed as AHA which was related to pemphigoid. Interventions: The patient had no response to the first-line treatment with corticosteroid and cyclophosphamide. Meanwhile, fatal hemorrhage occurred successively in thoracic cavity and right frontal lobe. rFVIIa and rituximab were administered. Outcomes: The patient survived from the life-threatening hemorrhage with a normal aPTT. His aPTT and FVIII:C level was normal during the follow-up of 6 months. Lessons: Rituximab and rFVIIa can play a critical role in rescuing AHA that is refractory to the first-line treatment.